This study evaluated whether inspiratory muscle training (IMT) could increase inspiratory muscle strength and endurance and exercise performance in 11 patients with cystic fibrosis (CF) with moderately severe airflow limitation. The IMT consisted of breathing through an inspiratory resistance (IR) for 15 min twice daily for 4 wk, preceded or followed by a 4-wk control period. After IMT, there was an increase in inspiratory muscle strength measured by maximal inspiratory mouth pressure at functional residual capacity: 74 +/- 18 cmH2O before training to 81 +/- 12 cmH2O after training, mean +/- 1 SD, p less than 0.025, and in inspiratory muscle endurance measured by the maximal IR tolerated for 10 min. Limitation of performance in the progressive exercise test was related to increased airflow limitation and possibly to poor nutrition. Mean exercise performance during progressive or submaximal exercise testing did not change after training. It is concluded that this form of IMT improved inspiratory muscle strength and endurance, but had little effect on exercise performance in patients with CF.